Adenosarcoma mülleriano de útero: un tumor infrecuente de difícil diagnóstico / Uterine müllerian adenosarcoma: report of a case and review of the literature

El adenosarcoma de útero es una neoplasia rara y representa aproximadamente el 8% de todos los sarcomas uterinos. Está relacionado con antecedentes de tratamiento con tamoxifeno y con radiación pélvica. La clínica más característica es la de una lesión polipoide recidivante, siendo habitual que la paciente ya haya tenido algunas biopsias previas por ese mismo motivo, en las cuales no haya podido llegarse a un diagnóstico acertado y definitivo. Incluso puede haber llegado a darse un diagnóstico erróneo, de los cuales el más frecuente es el de un pólipo cervical. La lesión consta de un componente glandular benigno creciendo inmerso en un estroma sarcomatoso. Presentamos el caso de una mujer de 53 años con una lesión polipoide uterina. Tras los estudios macroscópico, microscópico e inmunohistoquímico se llegó al diagnóstico de adenosarcoma mülleriano de útero. Se trata de una neoplasia de bajo grado y buen pronóstico, pero que recidiva en un alto porcentaje de casos (25-40%). El tratamiento es la histerectomía total simple con doble anexectomía, seguida o no de radioterapia postoperatoria, aunque en pacientes jóvenes, que no tienen el deseo genésico cumplido, al ser una neoplasia de bajo grado, se han descrito casos en los que se ha optado por un tratamiento conservador. De todas formas, no hay que olvidar que se trata de un tumor maligno y que también se han descrito casos de fallecimiento por esta entidad a causa de metástasis abdominales o de otro tipo, por lo que somos de la opinión de que la mejor y más segura opción terapéutica es la histerectomía (AU)

Uterine adenosarcoma is a rare neoplasm constituting only around 8% of all uterine sarcomas. This tumor is associated with tamoxifen therapy and pelvic radiation. The most characteristic clinical feature is a recurrent cervical polypoid lesion. Patients have often had previous biopsies for the same reason, but without an accurate diagnosis. Furthermore, a mistaken diagnosis may have been made, the most common being cervical polyp. Uterine adenosarcoma consists of neoplastic glands with a benign appearance and a sarcomatous stroma. We report the case of a 53-year-old woman with a uterine polypoid lesion. After macroscopic, microscopic and immunohistochemical studies, the diagnosis was a uterine Müllerian adenosarcoma, which is a low-grade neoplasm with good prognosis, but with a high percentage of recurrences (25%-40%). Treatment is simple hysterectomy with double adnexectomy, with the option of subsequent postoperative radiotherapy. However, because this tumor is a low-grade neoplasm, a more conservative approach has sometimes been adopted in some young patients without children. This tumor is malignant, however, and mortality from abdominal or other types of metastases has been reported. Therefore, we believe that the most appropriate and safest therapeutic option is hysterectomy(AU)

SEL1L expression in pancreatic adenocarcinoma parallels SMAD4 expression and delays tumor growth in vitro and in vivo.

Recent data suggest that SEL1L may play an important role in pancreatic carcinoma, similar to breast cancer, where the expression of SEL1L has been associated with a reduction in both proliferative activity in vitro and clinical tumor aggressiveness. To investigate this possibility, we examined the expression of Sel1L in a series of primary pancreatic carcinomas by immunohistochemistry and characterized the effects of Sel1L overexpression both in vitro and in vivo. In 74 pancreatic cancers analysed, 36% lacked Sel1L expression, although there was no significant correlation between the expression of Sel1L and any clinicopathologic parameter, including survival. However, immunohistochemical reactivity for Sel1L and Dpc4/Smad4 was concordant in 69% of cases (chi(2) test P&<0.004). Overexpression of SEL1L in stably transfected pancreatic cancer cells caused both a decrease in clonogenicity and anchorage-independent growth as well as a significant increase in the levels of activin A and SMAD4. When implanted in nude mice, Suit-2-SEL1L-overexpressing clones displayed a considerably reduced rate of tumor growth. Thus, it can be hypothesized that Sel1L plays an important function in the growth and aggressiveness of pancreatic carcinoma. Moreover, our data provide evidence that SEL1L has an impact on the expression of genes involved in regulation of cellular growth, possibly through the TGF-beta signaling pathway.

Extrauterine Müllerian adenosarcoma of the peritoneum with an extensive rhabdomyosarcomatous element and a marked myxoid change.

A case of extrauterine Müllerian adenosarcoma of the peritoneum in a 20-year-old woman is reported. The tumor was widely based on the abdominopelvic wall and there were no unusual features in the genital organs. The cut surface of the tumor showed a marked gelatinous appearance. The tumor was composed of an admixture of benign Müllerian-type epithelium and sarcomatous stroma. The predominant element of the sarcomatous area was rhabdomyosarcoma, which showed a close resemblance to well-differentiated embryonal rhabdomyosarcoma. In another sarcomatous area, fibroblastic cells without myoblastic properties diffusely proliferated in a marked myxoid background with some collagen bundles. Both the mitotic count and Ki-67 proliferative index of these cells were lower than those of rhabdomyoblastic cells. On follow up, the patient was disease free for 1 year postoperatively, without any subsequent treatment. The present case indicates that extrauterine adenosarcoma can also show histological heterogeneity as do uterine adenosarcomas. The remarkable myxoid change of this tumor seemed to be more largely due to a fibromyxoid element than a rhabdomyosarcomatous element, and the coexistence of the former may be related to the less aggressive behavior of this tumor.

Adenosarcoma mulleriano heterólogo en la adolescencia: presentación de un caso clínico y revisión de la literatura / Mullerian, heterologous, adenosarcoma in adolescence: one case presentation and literature review

Se presenta el caso clínico de una paciente de 16 años de edad, con diagnóstico histopatológico de sarcoma mulleriano mixto heterólogo, entidad poco frecuente en la adolescencia. Asimismo se realiza una revisión de sarcomas del útero, su epidemiología, clasificación, perfil clínico, pronóstico y tratamiento